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Archives of Clinical Gastroenterology

Tirotta Daniela* and Durante Vittorio

Case Report

Internal Medicine, Cervesi Hospital, Cattolica


(AUSL Romagna), Italy

Gastrointestinal Amyloidosis: An
Unusual Case Where ProteinLosing Enteropathy is Associated to
Malabsorption

Dates: Received: 30 April, 2016; Accepted: 24


June, 2016; Published: 25 June, 2016
*Corresponding author: Daniela Tirotta, Medicina
Interna, ospedale Cervesi di Cattolica (AUSL
Romagna), Via Beethoven 1, 47841, Cattolica
(AUSL Romagna), Italy, Tel: 0541/966291; Fax:
0541966290; E-mail:
www.peertechz.com
ISSN: 2455-2283

Abstract

Keywords: Gastrointestinal AL amyloidosis;


Enteropathy protein-losing; Malabsorption

Introduction: The systemic amyloidosis usually doesnt save the digestive tract, but this
involvement is present in approximately 60% of patients with AA Amyloidosis and only in 8% and 1%
of patients with AL Amyloidosis (respectively through biopsy and clinically). Beside gastrointestinal
involvement is rarely symptomatic and clinical events are not specific.
Enteropathy protein-losing is a rare condition caused by excessive loss of serum protein in
the gastrointestinal tract, resulting in hypoproteinaemia, edema, and, sometimes, pleural/pericardial
effusions. The diagnosis should be taken into account only when other causes have been excluded.
Gastrointestinal AL amyloidosis usually appears with constipation and mechanical obstruction/
pseudobstruction, on the contrary enteropathy protein-losing and malabsorption are rare.
Case report: We report the case of a 59-year -old patient, with AL amyloidosis, who suffered
from weight loss and ascites effusion related to an AL amyloidosis.
Discussion: The case is unusual, both because of the extension in the gastrointestinal tract
and for its unusual clinical presentation as enteropathy protein-losing associated with malabsorption.

Introduction
The systemic amyloidosis usually doesnt save the digestive
tract, but this involvement is present in approximately 60% of
patients with AA Amyloidosis and only in 8% and 1% of patients
with AL Amyloidosis (diagnosis respectively made through biopsy
and clinically) [1]. Beside gastrointestinal involvement is rarely
symptomatic and clinical events are not specific [2].
Enteropathy protein-losing is a rare condition caused by
excessive loss of serum protein in the gastrointestinal tract, resulting
in hypoproteinaemia, edema, and, sometimes, pleural/pericardial
effusions. The diagnosis should be taken into account only when
other causes have been excluded (malnutrition, heavy proteinuria,
and reduced protein synthesis due to liver diseases).
Gastrointestinal AL amyloidosis usually appears with constipation
and mechanical obstruction/pseudobstruction, on the contrary
enteropathy protein-losing and malabsorption are rare [1,2].

Case Presentation
A 59 years old man was admitted for loss weight, taste perversion,
diarrhea. Medical history:
A month earlier, due to an exertional dyspnea, he carried out
an ECG (right ventricular hypertrophy and previous anterior septal
ischemia)
He carried out an echocardiogram, positive for mild cardiac
hypertrophy (apical prevalence), left atrial enlargement, and small
pericardial effusion.

The Diagnosis. Physical examination showed basal pulmonary


obtusity, abdomen US confirmed ascites and pleural effusion.
Macroglossia was absent.
Blood tests showed low cholesterol levels (122 mg/dl total, 79 mg/
dl LDL, 29 mg/dl HDL, 70 mg/dl), hypoproteinemia (54 g/l protein,
64.1% albumin); hypogammaglobulinemia (9.6% gamma-globulins).
Thoracoabdominal computed tomography confirmed modest
ascites and lung interstitium thickening.
Because of gastrointestinal symptoms, ascites, hypoproteinemia
and low cholesterol levels (in absence of malnutrition, proteinuria,
and liver disease), we assumed the hypothesis of enteropathy
protein-losing. The cardiopathy and the hypogammaglobulinemia
encouraged us to think about lymphoproliferative disease.
Therefore, we planned an esophagogastroduodenoscophy
and a colonoscopy, with evidence of widespread gastrointestinal
amyloidosis.
In particular endoscopy showed erythematous striate in the
gastric antrum, wall thickening, erythema and erosions in the
second portion of the duodenum, extensive scarring of the whole
circumference in the proximal transverse.
Histological examination showed the homogeneous eosinophilic
material in duodenal and gastric withdrawals, in the vascular and in
the lamina propria, with epithelial erosions, finally deposits in the
vessel wall of submucosa of large intestine. No evidence of lymphoma.
Positive staining with Congo red.

Citation: Daniela T, Vittorio D (2016) Gastrointestinal Amyloidosis: An Unusual Case Where Protein-Losing Enteropathy is Associated to Malabsorption.
Arch Clin Gastroenterol 2(1): 050-052. DOI: 10.17352/2455-2283.000020

050

Daniela and Vittorio (2016)

Blood tests revealed mild proteinuria (0.19 g/24 hours), urine


Bence Jones positive, peripheral CD4/CD8 ratio inversion. Blood
tests also showed high pro-BNP (4829 ng/l) and positive troponin on
two points (63 ng/l). LDH and serum urea were normal.

obstruction (due to dysmotility ) in AL amyloidosis (39-50%)


[1,2].

bleeding (25-45 % of patients) due to ischemia, infarction,


vascular friability, mucosal lesions

Therefor it was planned a bone marrow biopsy, positive for


monotypic kappa myeloma, first pathological stage associated to
monotipicity for the light chain kappa immunoglobulin.

malabsorption (due to mucosal infiltration, pancreatic

In the context of vascular walls there is a deposition of amorphous


homogeneous substance and positive PAS: compatible with amyloid
(Congo red staining positive) (Figures 1,2).

Protein losing gastroenteropathy: diarrhea, ascites, edema,

Cardiac MRI confirmed typical altered kinetics of gadolinium.


Abdominal fat aspirationwas positive for amyloidosis.
Due to the presence of an unusual manifestation of an unusual
disease, the patient was sent to the Centre of reference for amyloidosis.

Discussion
Gastrointestinal tract involvement is almost constant in patients
with systemic amyloidosis (70-100% of patients), but a significant
number is asymptomatic [1-4]. Only 30-60% develop digestive
symptoms [5,6], therefore the diagnosis of digestive amyloidosis is
often difficult. In our case, in fact, it was made quite late.
Symptoms, if present, are aspecific:

diarrhea and malabsorption (due to rapid intestinal transit)


usually in AA amyloidosis, while obstruction/psudo-

insufficiency, bacterial overgrowth), loss weith, diarrhea,


steatorrhea
pleural/pericardial effusion, evidence of hypoalbuminemia

Macroglossia (25%)
Our case is characterized by rare clinical manifestation for
AL amyloidosis: a combination of enteropathy protein-losing and
malabsorption.
Enteropathy protein-losing is related to an increased capillary
permeability to plasma proteins, to exudation trough an inflamed
mucosa and, also, to the functional disruption of the lymphatic flow
in the bowel and mesenterium [7].
Gastrointestinal amyloidosis may be localized or widespread [4].
The most common sites of amyloid deposits are the veins and arteries
of the submucosa and muscularis propria. Also lymphatic vessels of
submucosal and subserosal are involved, as well as the muscularis
mucosae and, finally, mucosal or neuromuscular infiltration or
extrinsic autonomic neuropathy [1].
In AL amyloidosis polypoid protrusion, the thickening of the
valvulae conniventes are more common
In AA amyloidosis erosions, mucosal friability and granular
appearance are prevalent
In our case the macroscopic aspect is not usual and is more typical
for AA amyloidosis.

Figure 1: Gastric histological examination: homogeneous eosinophilic


material, attributable to amyloid perivascular and in the lamina propria, with
epithelial erosions. Positive staining witvh congo red in the polarized light.

In gastrointestinal amyloidosis endoscopic signs are various


and non-specific [4], 3/4 of endoscopic examination are normal [4].
Possible endoscopic aspects are: grainy appearance of the mucosa,
hematoma, submucosal polyps, thickening of mucosa, superficial
erosions or ulcerations; villous atrophy (rare).
Digestive amyloidosis has poor prognosis (specially for bleeding
complications and malnutrition). The median survival is 12 months
[4]. In the diffuse forms, the treatment is poorly codified [4].
Our case is emblematic for gastrointestinal amyloidosis:
clinical symptoms can be long silent; the endoscopic appearance is
varied and non-specific, therefore clinician should think about the
gastrointestinal histological examination.
The widespread involvement of the gastroenteric tract, the
enteropathy protein-losing are unusual for AL amyloidosis, but due
to the poor prognosis, it is necessary for the clinician to think earlier
about this diagnosis.

Figure 2: Intestinal histological examination: homogeneous eosinophilic


material, attributable to amyloid in the vessel wall of submucosa. Positive
staining with Congo red in the polarized light.

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Acknowledgements
To Giovanna Fabbretti, Anatomia Patologica of Rimini Hospital
(AUSL Romagna).

Citation: Daniela T, Vittorio D (2016) Gastrointestinal Amyloidosis: An Unusual Case Where Protein-Losing Enteropathy is Associated to Malabsorption.
Arch Clin Gastroenterol 2(1): 050-052. DOI: 10.17352/2455-2283.000020

Daniela and Vittorio (2016)

References
1. Camilleri M, Friedman LS, Grover S (2015) Gastrointestinal amyloidosis:
Clinical manifestations, diagnosis and management.
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3. Milovic V, Grand RJ, Lamont JT (2013) Protein-losing gastroenteropathy. Up
To Date.
4. Bchade D, Carmoi T, Algayres JP (2008) Amylose digestive (foie exclu).
EMC (Elsevier Masson SAS, Paris), Gastroentrologie 9-089-C-15.

5. James DG, Zuckerman GR, Sayuk GS, Wang HL, Prakash C (2007) Clinical
recognition of AL type amyloidosis of the luminal gastrointestinal tract. Clin
Gastroenterol Hepatol 5: 582588.
6. Repiso A, Valle J, Rodriguez-Merlo R, Munoz-Rosas C, Bolano F, et al.
(2003) Gastrointestinal bleeding and small bowel amyloidosis. Rev Esp
Enferm Dig 95: 578580.
7. Kawaguchi M1, Koizumi F, Shimao M, Hirose S (1993) Protein-losing
enteropathy due to secondary amyloidosis of the gastrointestinal tract. Acta
Pathol Jpn 43: 333-339.

Copyright: 2016 Daniela T, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and r eproduction in any medium, provided the original author and source are credited.

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Citation: Daniela T, Vittorio D (2016) Gastrointestinal Amyloidosis: An Unusual Case Where Protein-Losing Enteropathy is Associated to Malabsorption.
Arch Clin Gastroenterol 2(1): 050-052. DOI: 10.17352/2455-2283.000020

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